White Cell Disorders i

7/24/2019 White Cell Disorders i

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WHITE CELL DISORDERS I & II

Title: White Cell Disorders I & II

Date & Time: Monday, November 12, 2012 at 12 nooon (White Cell Disorers I)

Date & Time: Wednesday, November 1, 2012 at 12 nooon (White Cell Disorers

II)

!e"t#rer: The $atholo%y Team

QUIZBANK -- Blood & Lymph #'s 133-139, 178-333

INTROU!TION

"o $%ll ( )o )h%s m*)%*l +y )%m yo (l * l* lymph od ospl, o h*+ * p*)%) $%)h * *.om*l !B!/

0Lm%*s *d lymphom*s0 %s )h mos) d%((%2l) %) % d%2*l

4*)holoy 52p) (o loml* d%s*s/ "o 2*') l* %) %( yo * o)2o)%*lly *s% yosl(, 06hy0

"o * *l*dy (*m%l%* $%)h )h d+lopm) o( )h d%(() %ds o($h%) 2lls, *d )h lo2*)%os o( lympho%d )%ss )hoho) )h .odylymph ods, 6*ldy's %, 4y's p*)2hs, spl, l* *%$*ys/

T-2ll :os; )hyms, lymph od p**(oll%2l* 2o)5, spl%2$h%) plp * *)%ol

B-2ll :os; m%*l 2)s *d )h% m*)ls, spl%2 $h%)plp *) %)s m*%s

Amo 2%2l*)% lympho2y)s, 8


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I d%s2ss% d%s*ss )h*) *((2) m.s o( $h%) .lood 2lls % )hp%ph*l .lood, %) %s m2h mo s(l )o )*l *.o) AB@OLUT !LL!OUNT@ )h* 0p2)* 2o)s0/

O( 2os, yo 2* s)%m*) )h *.sol) 2o) .y ml)%ply% )h

)o)*l 6B! 2o) 5 )h = (o * p*)%2l* 2ll/

*l)hy *.sol) 2o)s;

B*soph%ls; ? ($- 1


3/105

A ood 0om*l *0 (o )o)*l $h%) 2o) %s D


4/105

!3, !D, !8, PT2R, T2R, T2R, ST!R, o)hs; )h mom*) T-2lls +*%os %ds

!1* T; som T-2lls, *ll L*h*s m*2oph*s

!E; m*)l 2ll lymphom*, m*y !LL

? !1< !ALLA; mos) B-2lls m*)l 2ll lymphom* %s *)%+

!1E; os) Rd-@). 2llsF som o)hs

? !19; B-2lls, .) o) pl*sm* 2lls

? !>>; mos) B-2lls B 2p)o

? !3D ; p%m%)%+ .lood 2lls -- *) (o 2o)% 0.l*s)s0 %lm%* C plm%*

!DE 02ommo lo2y) *)%0 C L!A; *ll $h%) 2lls? 52p)%o; Rd @). 2lls *d som lm%*s

? !E; os) 2os%s)) m* (o )h *)*l-%ll lympho2y)s,o-B, o-T 2lls m*% p m*y. 1; 1D>E, >


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* 2lo*l I !lo*l%)y (o B-2lls, T2R-*mm* !lo*l%)y (o T-2lls/

2y2l% 1; m*)l 2ll lymphom* s)*%s s)oly

2y)opl*sm%2 I; pl*sm* 2lls

? osp2%(%2 s)*s; moo2y)s

M2 2p)o; B-2lls, moo2y)s

TRA4; h*%y-2ll lm%*

LA-CR CI*; L*h*s 2lls *d o)h *)%-ps)%m*2oph*sF som o)h 2lls

lyso:ym; moo2y)s

? *lph*1-*)%2hymo)yps%; moo2y)s

y)hoph*o2y)os%s; moo2y)s

mylo-po5%d*s; *lo2y)s

? @d* .l*2; *lo2y)s

? 2hloo*2)*) s)*s; )oph%ls, .*soph%ls, m*s) 2lls

pl*)l) m*s; m**yo2y)s

? 4A@ d%((sly; y)ho2y)s, m**yo2y)sCpl*)l)s

? 4A@ 2hs 0.lo2s0; %mm*) lympho2y)s o lm%*

@-1


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? Th h*s . )*l o( d%*os% *d 2l*ss%(y% lymphom*s.*sd o l%))l .%ops%s lss %+*s%+ )h* )*% o) * $hollymph od/ As yo'd 5p2), lss )h's * *sy )*dm*(%d% o )$o m*)l-2ll lymphom*, T-lympho.l*s)%2 lymphom*,%) 2*') . do l%*.ly Am/ / !l%/ 4*)h/ 1>8; D7D, >


7/105

@p*2-o22py% ls%os 0myloph)h%s%2 *m%*s0

@ol%d 2*2s

*lom*s

m*)olo%2 m*l%*2%s )h*) sppss*lopo%s%s %//, som lm%*s *d lymphom*s

NA po.lms

!*2 2hmo)h*py

R*d%*)%o s%2ss

0Th m*lo.l*s)%2 *m%*s0

d%)*y 2y2l%2 J/ 3 $/, s+F dom%*) m)*)%os*lly % )h LA> l*s)*s , mol2l* .%oloyBlood 9>; >>9, 1998F o 2*s %s m)*)d)oph%l l*s)*s )h*) %)sl( d*m*s )h 2lll*m*2h%yF *lso N*)/ )/ 3E; 9


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s)*/ Th s*l 2*s o( +y lo$ *.sol))oph%l 2o) *) .%)h *d *()/

Id%op*)h%2

? Th l*. m*2h% d%d') 2o) )hm/

Th .lood s*) % TA )oo lo *d )h)oph%ls s)2 )o)h/

Th p*)%) h*s o o( lm%*s Cplm%*s % $h%2h )h )oph%ls do')m* m2h mylopo5%d*s, *d )h m*2h%sd )h mylopo5%d*s *2)%o )o 2o))oph%ls

!@@ @TRU!TION OM NUTRO4IL@

A)o%mm *, )h% o( lps

ypspl%sm s .lo$

@Js)*)%o % * *p%dly-o$% *.s2ss

Id%op*)h%2

RU@; Th m2h*%sms * )yp%2*lly o.s2 A/ I)/d/ 1D; E7, >


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d*pso

d%pyo

m)h%m*:ol

po2*%*m%d

popyl)h%o*2%l

%)5%m*.

sl(*s*l*:%

)%2lop%d%

4R@ONAL-TRIIAL

@om popl o( A(%2* ds2) popl Vs) h*+

sl%h)ly lo$ )oph%l 2o)s

@om $om ) * m%ld )op%* *od )h%p%ods

ARANULO!"TO@I@ %s * )%m-hood m%som (o)op%* s((%2%)ly s+ )o p) * pso *) %s (o s%os


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%(2)%o %//, )oph%l 2o)s o( 1


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o $%dsp*d )%ss 2os%s (om *y 2*s

do') (o) sy *d myo2*d%*l %(*2)s

o l*) p*2y 2ommo, m%ld

o *lly .*d 02oll*-+*s2l* d%s*s0

o Vs) pl*% 0s)ss0, *s*, *dCo phys%2*l p*% -m*%*)s)oph%ls

l2o2o)%2o%ds *d p%ph% do )h s*m )h%F

l2o2o)%2o%ds *lso p+) )oph%ls (om )%)%sss

NOT; Typho%d p*)%)s *d som sp-sp)%2 p*)%)s m*y.2om )op%2 .2*s *lopo%s%s %s sppssd*dCo *ll )h )oph%ls h*+ m%*)d (om )h .lood/ B$*o( ly% o $h%) 2o) *s yo 2h%( m* (o %(2)%o

NOT; Th sp-s%2, sp)%2 p*)%) %s l%ly )o h*+ TOI!RANULATION 5)*-pom%) *:oph%l%2 *ls,!"TO4LA@I! A!UOL@ 0(om do% *ll )h*) ph*o2y)os%s0,*dCo OL BOI@ oh dopl*sm%2 )%2lm m*)s/

By 2o)*s), %( )h )oph%l 2o) s%mply %ss (om *2) p*%*d 0s)ss0, )h $%ll . o )o5%2 *l*)%o, +*2ol%:*)%o, ol() sh%()/ o *.o) )hs % 0!l%%2*l 4*)holoy0/

{13646}ohl .ody{13661}ohl .ody{16213}ohl .ody

? M) p*)holo%s)s; Th l*)) )$o 0ohl .od%s0 *(*sF )hy * (om 2*ss o( *y-l%'s s*y 0h-

A"-l0 sm%-d%s*s, * *)osom*l dom%*) )*%) $%)h)oo-($, )oo-.% pl*)l)s *d lo)s o( 0ohl .od%s0 *d .%*lsF )h )oph%ls (2)%o om*lly/ *y-l%0ohl .od%s0 * *2)*lly o-ms2l myos% A, m)*)d % *y-l%; N*)/ )/ >; 1


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NOT; LMT @IMT (s )o ps2 o( %mm*) $h%) 2lls0.*ds0 % )h p%ph*l .lood, %//, )hy' .% mo.%l%:d*ly (om )h .o m*o$/ To )ll * 5)m 2*s 6B!Gp )o1


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DED, >


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+%s2*l l*+* m%*s do *d 2*) od$oms

2)*os l*+* m%*s do *d 2*) hoo$oms

*lly mos) *yF .) o)o%osly old )h*py (o *)h%)%s, $h

os%oph%l%* %s *lmos) 5p2)d

od%'s d%s*s * l* m%o%)y o( 2*ss

!h-@)*ss * +*s2l%)%s, o() $%)h *lom*s, s*lly $%)h AN!AF%)'s o) 2l* $h)h )h%s %s * sp**) d%s*s, o s%mply )h $*y6's C poly*)%)%s m*%(s)s % (ols $%)h *ll%s

m*)%)%s hp)%(om%s

M*m%l%*l hypos%oph%l%* lo2s o$, *)osom*l dom%*), m%ld;Blood 1


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? m*s)o2y)os%s $%)h os%oph%l%* mol2l* s%*) o$, spos)o %m*)%%.Cl+%2 l%ly

? T-2ll opl*sms m*% %)l%-E; N 3D1; 11D1, 1999

? 0!lo*l os%oph%l%*0 -- MI41L1-4MRA (s%o

? os%oph%l%2 lm%* -- loos l% !L $%)ho) poo( o( 2lo*l%)y

NOT; I )h d+lopd $old, *mo 2l%%2*lly h*l)hy p*)%)s $%)h%sol*)d l+*)d os%oph%l 2o)s, yo $%ll o() o) (%d )h 2*s/

NOT; I d%d !B!'s (o y*s o md%2*l s)d)s, m*y o( $hom h*+h*y (+, )2/, *d h*+ + (od o $%)h * l+*)d os%oph%l2o)/

NOT; Rmm. )h*) os%oph%l%2 2o)s * p % )h *()oo *ddo$ % )h mo% .2*s )h mo%'s 2o)%sol s sppsss)hmF I'd ss) )*% * s%os loo *) * *.sol) os%oph%l 2o)o+ 3E< o so % )h mo%, *d o+ E< % )h *()oo/

NOT; Th 0Lo((l's os%oph%l%20 po.lms * * 2%os, m%5d-.*o( d%s*ss $%)h 52ss%+ m.s o( os%oph%ls % +*%os )%sss)h*) 2*s )%ss d*m*/

Th mos) 2ommo (om %s po.*.ly 2*sd .y * .% opl*smo( som so), h%dd som$h, $%)h * (s%o 2*lldMI41L1-4MRA !*2 11


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{14099}os%oph%l%2 lo2y)s .((y 2o*){09207}os%oph%l *l $%)h 2ys)*l l2)o m%2o*phsF )hs 2ys)*ls$%ll 2om.% )o (om l* !h*2o)-Lyd 2ys)*ls d som 2od%)%os

os%oph%l%*

T5) *d pho)om%2o*phs/ N%2/m* 4*)holoy %%)*l Im* *lly

LOT@ OM ONO!"T@;

)ypho%d (+

.*d *lom*)os po.lms

TB

.2llos%s!oh's d%s*slposydp (%s*2o%dos%so)hs

? 2ho%2 *)o%mm d%s*s

hm*)o%d *)h%)%s %s $o)h mm.%

? %2))s%*l d%s*s o()F d (l*

? d%ssm%*)d 2*2 o22*s%o*lly

LOT@ OM L"4O!"T@;

0%(2)%os moo2los%s0 s .lo$

$hoop% 2oh 0p)ss%s0F l%))l 2l*+d lympho2y)sF )h )o5% ps

)h T-2lls (om hom% )o lympho%d )%ssF Am/ / !l%/ 4*)h/ 11D; 3E,>


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0)*s%) s)ss lympho2y)os%s0 *.sol) 2o)s D


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%mmo.l*s)s -- .% 2lls, .%) 2lol%, pl)y o( .l-s)*%%2y)opl*sm

4o.*.ly mo %mpo)*) % spo))% %(2)%os moo *d )ll% %)(om lm%* $h yo' * .% %s )h*) $h )h *.2h o( 0*)yp%2*l lympho2y)s0, o )$o loo )h s*m/

I(2)%os moo2los%sBlood p%2)

6.4*)h 4ho)o

I(2)%os oo2los%sT5) *d pho)om%2o*phs/ N%2/

m* 4*)holoy %%)*l Im* *lly

LOT@ OM BA@O4IL@;

2ho%2 myloos lm%*

o)h 02ho%2 mylopol%(*)%+ d%sods0

poly2y)hm%* +*

? p%m*y hmoh*%2 0ss)%*l0 )hom.o2y)hm%*

? spposdly % lo)s o( o)h )h%sF )h%s $%ll o) . %mpo)*)2l%%2*lly/

NOT; No o( )hs 02l*ss%2 (%d%s0 %s %)h p*)%2l*ly ss%)%+, op*)%2l*ly sp2%(%2, (o *y p*)%2l* d%s*s/ Us )h%s %(om*)%o %)h s))% o( )h 0$hol pso0/

O NUTRO4IL@;
http://library.med.utah.edu/WebPath/HEMEHTML/HEME013.htmlhttp://library.med.utah.edu/WebPath/HEMEHTML/HEME013.htmlhttp://library.med.utah.edu/WebPath/HEMEHTML/HEME013.htmlhttp://www.microscopyu.com/galleries/pathology/index.htmlhttp://www.microscopyu.com/galleries/pathology/index.htmlhttp://www.microscopyu.com/galleries/pathology/index.htmlhttp://library.med.utah.edu/WebPath/HEMEHTML/HEME013.htmlhttp://library.med.utah.edu/WebPath/HEMEHTML/HEME013.htmlhttp://library.med.utah.edu/WebPath/HEMEHTML/HEME013.htmlhttp://www.microscopyu.com/galleries/pathology/index.htmlhttp://www.microscopyu.com/galleries/pathology/index.htmlhttp://www.microscopyu.com/galleries/pathology/index.html


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6 h*+ *l*dy m)%od !RONI! RANULOATOU@ I@A@,* pooly-*md op o( d(2)s % )h *.%l%)y o( )oph%ls )o %ll

2ommo .*2)%*, $%)h )h m*2oph*s d% )o .2om %+ol+d*s $ll/

Th mos) (*m%l%* %s 0-l%d 2ho%2 *lom*)os d%s*s0,$h%2h h*s o$ . 2d .y )h*py N*)/ d/ 1>; D


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most o# the rest! Don5t miss it!

6 A similar, than"#ully-rare periodi #e$er

syndrome is aused 1y a mutation in the

TNF-reeptor 3TNFR+4) 7lood +(0) +/(,((8! 9et another is aused 1y mutated

ryopyrin 3inludes serious neurologi

pro1lems anti-interleu"in-+ treatment1rings a1out #ull resolution) Neurology *:)

+8*, (+(4!

"o 2*ll !IAK-IA@I @"NRO, % $h%2h )h * s+*lpo.lms $%)h o*ll mm.* sy)hs%s/ sy)hs%s/

N)oph%l lysosoms * l* *d pom%) )hy (s $%)h*2h o)h *d do o) (s $%)h ph*osoms, so )h's poo.*2)%*l %ll% *d * lo) o( %(2)%os/

l*osoms do') (om poply, so )h %s p*)%*l *l.%%sm/

Th l*2 o( pl*)l) ds *ls sl)s % * .ld% )d2y/

? Th h*s . 2lod L"@T, lysosom*l )*((%2 l*)o/os) o( )hs p*)%)s o o )o d+lop * l)h*l o-opl*s)%2

hyppl*s%* o( )h lympho2y)s/ *o$ C s)m 2ll )*spl*) %so$ o)% *d p+)s )h%s/ R+%$ Blood 9E; 979, >


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!! pho)o

NORAL L"4 NO ANATO"

L"4 NO@ * so() %//, )%2l%-(*m$o o+o%ds, p )o *.o) >2m % h*l)h/ A(() lymph*)%2s p)*) *d )*+l $%)h% )h%2*psls m)*s)*)%2 2*2 (%s) s)s p h/ A()$*ds, lymphp2ol*)s )hoh )h 2o)5, *d )h )h mdll*, l*+% .y )hh%lm/

6%)h% )h 2o)5, )h * *lly som m%*l 2)s0lympho%d (oll%2ls0, s%)s o( *2)%+ly-pol%(*)% B-2lls/ *2hm%*l 2) %s sodd .y * m*)l o( s)% B-2lls,$h%2h * % ) sodd .y 0p**(oll%2l*0 T-2lls/ I( )h %s

o *)%%2 s)%mls, yo'll s oly 0p%m*y (oll%2ls0 o( slpyB-2lls % )h 2o)5/

Th 5) )%m yo ) )o loo *) * m%*l 2) d )hm%2os2op, 2h2 o) )hos pol%(*)% B-2lls/ ThsJ2 (om sm*ll B-2ll )o pl*sm* 2ll %s %)s)% *ds % mos) h%s)oloy 2oss/ "o'll d )o o$ )h%s)o ds)*d 2l*ss%2*l *22o)s o( lymphom*s;

Rs)% sm*ll B-lympho2y)

@m*ll 2l*+d 02l()d0, %//, (oldd-2ls B-lympho2y)

L* 2l*+d B-lympho2y)

@m*ll o-2l*+d B-lympho2y)XNOT; Th%s 2ll %s *s l* *s * l* 2l*+d B-lympho2y)Y

L* o-2l*+d B-lympho2y)

B-%mmo.l*s)

moy B-2lls / / *d / / 4l*sm* 2lls
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6%)h% )h mdll*y 2ods, 5p2) )o s * m%5 o( B- *d T-2lls *dpl*sm* 2lls/ Th s%so%ds * l%d .y (%5d ph*o2y)s/

sp%) )h l*) p%2)s % h%s)oloy .oos, lymph ods *sldom 0om*l0, sp2%*lly % *dl)s/

L"4ANITI@; I(l*mm*)%o o( )h lymph ods

A!UT L"4ANITI@ ds2%.d % 0B% Ro..%s0 %s o) m2h mo)h* )h hyppl*s%* % * *2)%+ od/

Lo2*l%:d lymph*d%)%s %s mos) o() d )o * .*2)%*l %(2)%o% )h ** d*%d .y )h lymph od/

R*lly .*d 2*ss h*+ polys *d + *.s2ss (om*)%o

$%)h% )h ods/ Th d sl) $%ll . * s2*d-p lymphod/ "o h*+ o o mo/

*l%:d lymph*d%)%s ss)s * sys)m%2 +%*l %(2)%o/

0s)%2 *d%)%s0, o() %d%s)%%sh*.l (om *2)*ppd%2%)%s, %s 2*sd .y "s%%* )o2ol%)%2*/

A2) lymph*d%)%s, s%2 %) 2oms p sddly *d s))2hs)h 2*psl, %s l%ly )o m* )h od )d/

!RONI! NON-@4!IMI! L"4ANITI@ (*lls % o o( )hd%s)%2)%+ p*))s/

MOLLI!ULAR "4R4LA@IA %//, lo)s *d lo)s o( .% (oll%2lssl)s (om los)*d% 2o)*2) $%)h o*%sms o 0o)h )h%s0)h*) s)%ml*) )h B-2lls/ I( ppl5d, )h% o(;

)o5opl*smos%s ? loo (o m%%-*lom*s )o2h% )hm%*l 2)s *) )h% ds, )h%s %s spposdly

p*)hoomo%2F som o( )hs * ops o( m*2oph*sFsom * .% 0moo2y)o%d B-2lls0 sp2%*lly % )hmdll*

hm*)o%d *)h%)%s o() lo)s o( pl*sm* 2lls


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syph%l%s pl*sm* 2lls % )h mdll*, m%%-*lom*s,sp%o2h)s

AI@-l*)d 2ompl5 C ps%s)) *l%:dlymph*dop*)hy o( I %(2)%o/

2ommo +*%*.l %mmod(%2%2y %((2)%+ B-2ll*2)%+*)%o

{36371})o5opl*smos%sF m*y .s % * 2ll{40654})o5opl*smos%sF )%ss *2)%o l*m-loo% *lom*s

@yph%l%s % * lymph od

")** Ts)sm%

To5opl*sm* lymph*d%)%s

")** Ts)sm%

Moll%2l* hyppl*s%*

6.4*)h 4ho)o

Moll%2l* hyppl*s%*

6.4*)h 4ho)o

Moll%2l* hyppl*s%*

6.4*)h 4ho)o

I lymph*dop*)hy

")** Ts)sm%

4ARA!ORTI!AL L"4OI "4R4LA@IA %//, lo)s *d lo)s o(lympho2y)s, %2ld% )d-o os, % )h T-2ll %os o( )h2o)5 -- o() *s%s) )o 2o%: )h )h ps2 o( pom%).lood +ssls sl)s (om los)*d% 2o)*2) $%)h o*%smso 0o)h )h%s0 )h*) s)%ml*) )h T-2lls/ I( ppl5d, )h% o(

%(2)%os moo2los%s, !

phy)o% 0%l*)%0 5pos

$%d *2)%os )o * +*22%

%(2)%os moo2los%s (*m%ly )hos *y T-%lls, )2/F? 5p2) )o s lo)s o( .% lympho%d 2lls $%)h p*l
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2y)opl*sm, pl)y o( 2os%s *d m%)o)%2 (%sF s *y! 2llsF )h%s 2* . * *l (ool (o lymphom*, s

A2h/ 4*)h/ L*./ d/ 117; >9, 1993

lps loo (o (* +*s2l%)%s *d + %os o(

%(*2)%o

@INU@ "4R4LA@IA (omly 0s%s h%s)%o2y)os%s0F %//,s%so%ds $%)h s$oll do)hl%*l 2lls *d lo)s o( h%s)%o2y)s/ I(ppl5d, )h% o(;

ods d*%% * 2*2 .y o m*s sp2%(%2

? ods %V2)d $%)h pm*) *d%oloy 2o)*s) md%m0lymph*%o*m dy0 o (o som *so 2o)*%%m%*l o%l 0olo*lom*s0

6h%ppl's d%s*s 6h%ppl 2lls

? !*s)lm*'s %*) *%o(oll%2l* lympho%d hyppl*s%* (*.yod yo l*% o.V2)%+sF N 33, 199DFo 2*s sp2%*lly $h ml)%(o2*l *d %2h % pl*sm*2lls sms )o . hps 8 C K@ Am/ / 4*)h/ 1E1;1E17, 1997F 6s)/ / d/ 17; 38, 1997F Blood 93; 3D3,1999/ I) %s * (*mos 2*s o( p**opl*s)%2 pmph%sL*2) 33; E>E, >


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6 ?sinus histioytosis with massi$elymphadenopathy and lymphoyte

emperipolesis? 3Rosai-Dor#man disease4) a

presuma1ly $iral, generally sel#-limited semi-disease o# young people! 7- and T-ells li$einside marophages 3?emperipolesis?4, whih

in turn light up with +(( and #or lipid!

There is a more ominous eBtranodal #orm aswell! No one understands it!

Ros*%-o(m*@1


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6ll-m*d *lom*s $%)h NOTIN ls * po.*.lys*2o%dos%s/ Also mm. !oh's, .yll%os%s, *d odsd*%% od%'s d%s*s/

*lom*s $%)h 4U@ % )h% 2)s * po.*.ly 2*sd .y o

o( )h (ollo$%; 1 lympho*lom* +m , > 2*) s2*)2h(+, 3 .2llos%s, D pl* , E )l*m%* , l*ds-ml%o%dos%s, *d 7 o)h ys%%* %(2)%os/ I( yo 2* (%d oo( )hs, 2os%d 8 -l%d 2ho%2 *lom*)os d%s*s)h )oph%l dys(2)%o po.lm/

? *lom*s $%)h 2)*l 2os%s $%)h m2h *yoh5%s .) ops; K%2h%-MV%mo)o/ @ .lo$/

T.2los lymph*d%)%s*) l*.ls

Rom*%* 4*)holoy A)l*s

B*)ollos%s!*) s2*)2h (+

")** Ts)sm%

"o h*+ po.*.ly *l*dy s )h 6*)h%-M%ldy %*) 2lls o(

m*sls $%)h% m%*l 2)s/ Thy sho$ +*%*.l %mmolo%2m*s -- B-2ll, T-2ll, *dCo dd%)%2 m*2oph*s/ @om h*+%)*2l* m*sls +%s %2ls%osF som do o)/

? KIKU!I-MUIOTO N!ROTIZIN I@TIO!"TI!L"4ANITI@ / Am/ A2*d/ m/ E9; 13


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L"4ANO4AT" %s * 2l%%2%*'s $od (o * .% lymph od/

NON-OKIN'@ L"4OA@; By d(%%)%o, moo2lo*l, m*l%*) )moso( )h B- o T-2lls, *d o) o( pl*sm* 2lls, *d o) od%'s d%s*s/ By2s)om, so() )mos o( moo2y)s * %2ldd h .2*s )hy loos%m%l*/

Ths * )h 2ommo p%m*y )mos *%s% % )h lympho%d )%sslymph ods, )os%ls, *do%ds, spl, 4y's p*)2hs, o-p%)hl%*l)hyms *d )h * som sp2%*l 2*ss/ 6 2o+ !N@ lymphom*s% )h 0o0 s2)%oF )h o)loo (o p%m*y !N@ lymphom* %s s)%ll0d%sm*l0; !*2 11


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/ 6*o2's !oll2)%o

M!! lymphom*4%))s.h 4*)holoy !*ss B-2ll lymphom*4%))s.h 4*)holoy !*ss

%((s l* 2ll lymphom**)

4%))s.h 4*)holoy !*ss

B-2ll lymphom*L* 2ll


Moll%2l* lymphom*, spl

AMI46%%md%* !ommos

Th o-od%'s lymphom*s * * s.V2) o( p%*l(*s2%*)%o (o p*)holo%s)s/ *% )h d%*os%s 0.% om*l%*)0 %s o() )oh, *d 2l*ss%(y% )h o-od%'slymphom*s h%*() 0lymphom*s0 $*s * m*Vo %)*)%o*l2omp)%)%+ spo) )hoh )h 198


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RUL; os) o-od% lymphom*s * som$h*) mo 2ommo %m, $%)h )h mos) poo2d d%((2 po.*.ly .% T-lympho.l*s)%2 lymphom* G >;1/

RUL; Bl*2s *d 2h%ld *lmos) + ) odl* lymphom*s/

RUL; A ($ sp2%(%2 lymphom*s h*+ o o mo sp2%*l %s (*2)os%//, hl%2o.*2) 2*ss )h ALT lymphom* o( )h s)om*2hF l))op*)hy 0sp0 2*ss * 2%os p*% o( T-2ll lymphom*s;*s)o)/ 13>; 19, >


31/105

891, 1998, O22p/ +%o/ d/


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RUL; Th lo$ )h *d o( )h lymphom*, )h OR l%ly )h .om*o$ %s )o . %+ol+d *) )h )%m o( d%*os%s/ 4**do5%2*l, o

RUL; Lymphom*s )d )o sp*d )o s%)s *22od% )o )h% B-2ll o T-2ll o%%/ B-2ll )mos o )o )h m%*l 2)s, )h% m*)ls, *d

)h o)s%ds o( )h spl%2 $h%) plp/ T-2ll )mos )o )h *)%omd%*s)%m, p**2o)%2*l %os o( ods, %s%ds o( spl%2 $h%)plp, )2/ @% lymphom*s * s*lly o( T-2ll o%%/

RUL; Th m*l%*) 2lls o( lymphom*s * OR %(om )h* )hm%5 o( 2lls om*lly s % lympho%d )%ss, *d )hy 2*p%)l*)som ph*s % )h l%( h%s)oy o( %)h om*l B-2lls o T-2lls/ o')5p2) )o s m2h 02y)olo%2 *)yp%*0 % * lymphom*/ Rmm. )h*))h om %s s*lly o) ds)*.%l%:d % lymphom*s/ sp2%*lly,%mmo.l*s)%2 lymphom*s 2* loo p))y $%ld/

RUL; Lymphom*s )h*) o$ *s odls $%)h% * lymph od 0)y% )o. m%*l 2)s0 * 2*lld NOULAR o MOLLI!ULARsyoyms/ Thy * *l$*ys o( B-2ll o%%, *d )h lymphom* 2lls$%ll 2losly sm.l o o( )h (oms % )h sJ2 (om s)% B-lympho2y) )o pl*sm* 2ll/

{23581}odl* lymphom*

Nodl* lymphom*

6.4*)h 4ho)o

Nodl* lymphom*

KU !oll2)%o

RUL; Nodl* lymphom*s )d )o . %dol) ls%os $%)h *)*lh%s)o%s )h*) * l*)%+ly *((2)d .y 2l*ss%2*l 2hmo)h*py/%s)o%2*lly, )hy h*+ . %2*.l, )hoh )h%s sms )o .2h*%/ *2h odl* lymphom* h*s * .)) poos%s )h* %)sd%((s 2o)p*), *d %s l%ly )o )*s(om %)o %) soo o l*)/ Th%s

m*s ss, s%2 (oll%2l (om*)%o %s * s% o( ood d%(()%*)%o/Lss o(), * odl* lymphom* )*s(oms %)o * d%((s l*-2ll o%mmo.l*s)%2 lymphom*/

? A lymphom* $%)h )$o d%(() mopholo%2 *pp**2s *d)%2 2los %s * 02ompos%) lymphom*0/ Th%s %s (*%ly 2ommo,*d m*y ps) )*s(om*)%o o( o lymphom* %)o *o)h
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.y *dd%)%o*l m)*)%os, o )$o sp**) m*l%*) )mos/))% %) $od o); Am/ / !l%/ 4*)h/ 99; DDE, 1993F Am/ /4*)h/ 1ED; 18E7, 1999F N3D1; 7D, 1999/ Th mos) (*m%l%*!LLC6LL m%5d % $%)h (oll%2l* lymphom* %s )$o d%(()2lo*l )mos Am/ / !l%/ 4*)h/ 137; D7, >/

RUL; os) odl* lymphom*s o( *ll %ds (*) o o( )$o2h**2)%s)%2 )*slo2*)%os, %)h )11F1D o )1DF18/ *2h %+ol+s)h %mmolo.l% h*+y-2h*% %o o 2homosom 1D/ Th%s %s.oh) %)o 2o)%%)y %)h $%)h )h .2l1 C 4RA C 2y2l% 1 o2oo 2homosom 11 o )h .2l-> o2o o 2homosom 18/

? A m*Vo%)y o( *dl)s h*+ som )1DF18 lympho2y)s o .o*d %(yo loo h*d; N 3E; 7D1, >


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*m 4@UOL"4OA/ %s)%%sh% )hs (om *l lymphom*s%s * 2h*ll/

Also mm. )h*) 2)*% *)o%mm d%s*ss (*) h*+ypoly2lo*l lympho%d %(%l)*)%o o( s*l%+*y l*ds @Vo's,

)hyo%d *sh%mo)o's, %sl)s )yp I d%*.)s, o %dys*)o%mm %)s)%)%*l ph%)%s/

? Mo som *so, Lym d%s*s pod2s psdolymphom*s% )h * lo.s/ No o h*s * 2l $hy/

RUL; 4*)holo%s)s )y% )o d%s)%%sh m*l%*) lymphom*s (om.% lymph od hyppl*s%*s *d psdolymphom*s p*y sp2%*l*)))%o )o;

1 MMA!NT OM T NORAL L"4 NOAR!IT!TURF

> !LL UNIMORIT" 0moo)oy0, ss)s lymphom*, .)+ (oll%2l* lymphom*s * %(%l)*)d .y )h s*m .% 2lls*s o$ % * m%*l 2)F

? 3 4s2 o( m*2oph*s l*d $%)h 2l* d.%sTINIBL BO" A!RO4A@, * s% )h*) )h po2ss %sITR .% OR * h%h-*d lymphom*, .2*s % lo$-

*d lymphom*s yo $o') s m2h *pop)os%sF

D 6%dsp*d .2l-> po)% s)*%% %s * p))y ood s% )h*))h%s %s lymphom*/

T%%.l .ody m*2oph*s

6.4*)h 4ho)o

E A@!ULAR 4ROLIMRATION $ +ssls ss) )h

po2ss %s .%, *dF

INA@ION o( sod% )%ss 02*psl* )*sss%o0,ss)s lymphom*/
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7 N!RO@I@ *p*) (om *pop)os%s %s 2ommo % somlymphom*s, *d o( 2os % 2o)%:% %(2)%os, .)2ommo % d%((%2l) .% ls%os/

8 I( 0(oll%2ls0C0odls0 * ps), )h AB@N! OM A

ANTL o( sm*ll lympho2y)s *od )h l%h) s%d o( )h (oll%2lss)s m*l%*2y/

? I AI@, m*)ls * l%ly )o *.s)/ 6hy

9 Tod*y's p*)holo%s), *s% 0Is )h%s lymphom*0, .%s *s(ollo$s;

I( %) %s *pp*)ly m*d o( sm*ll lympho%d 2lls, )hp*)holo%s) s)*%s (o *pp* *d l*m.d* moo2lo*l%)y %s

lymphom*, poly2lo*l%)y %s o-m*l%*), *d * .2l> s)*%%( )h * odls pos%)%+ s)*%% %d%2*)s lymphom*/

I( %) %s *pp*)ly m*d o( l* lympho%d 2lls, )hp*)holo%s) $%ll od * !DE lo2y) 2ommo *)%,pos%)%+ % lymphom*s, * ($ o)h lympho2y) m*s,2y)o*)%s *)%+ % lymphom*s, *d * ($ ml*om*m*s *)%+ % lymphom*s/

Lymphom* % lymph od

I+*s%o o( sod% (*)Tom m*'s @%)

1


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RUL; os) lymphom*s od%'s *d o-od%'s m*y 2*s*l%:d dys(2)%o o( .% B-2lls hypo*mm*lo.l%m%*,$%)h sl)% )d2y )o %(2)%o/

!LA@@IMI!ATION @!@;

Ayo s% )h )ms 0lymphos*2om*0, 0%*) (oll%2l*lymphom*0, o 0)%2lm 2ll s*2om*0 % )od*y's md%2% %s)%.ly o) o( d*)/

? T 19 RA44A4ORT !LA@@IMI!ATION %s *2h*%2 .) s)%llpopl*/ I) $*s .*sd o 2)*% %2o2) .) o2-s(l*ssmp)%os *.o) )h *) o( )h 2lls s % )hs ls%os;

06ll-d%(()%*)d lympho2y)0/// loos l% * om*l

s)% lympho2y)

04ooly-d%(()%*)d lympho2y)0/// dos') loo l% *om*l s)% lympho2y), .) %s sm*ll )h* *do)hl%*l 2ll

0%s)%o2y)0/// .% )h* * do)hl%*l 2ll, *d h*s lo)s o(2y)opl*sm

0Ud%(()%*)d 2ll0/// .% )h* * do)hl%*l 2ll, *d

h*s oly * l%))l 2y)opl*sm

Lymphom*s $ ()h s.-d%+%dd %)o 0odl*0 *d0d%((s0, dpd% o )h% o$)h p*))/ sp%) %)sl%m%)*)%os, )h R*pp*po) sys)m $*s s(l *s lymphom*s$ .% so)d o)/

? T 197D LUK@-!OLLIN@ !LA@@IMI!ATION $*s .*sd op%m%)%+ %mmo)yp% *d 2los 5*m%*)%o o( )h mopholoyo( )h 2lls, $h%2h $ 2omp*d )o )hos % )h 2)s o(

om*l m%*l (oll%2ls/ A2)%+*)d-)yp B-2lls (om sm*ll-2l*+d )hoh l*-o2l*+d 2lls $ *ppop%*)ly 2*lldMOLLI!ULAR !NTR !LL@/ + mo 52%)% $ )hIUNOBLA@T@, +y .% od 2lls $%)h +y .% od 2l%.*% % )h% 2)s * s%l +y .% 2lols I 2*ll )hm0y.*ll 2lls0/


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? T 198> 6ORKIN MORULATION $*s * 2osss o(5p)s .*sd oly o mopholoy/ I) $od %2ly )%l %) $*sspsdd .y )h R+%sd op*-Am%2* sys)m/ "o'll s)%ll(%d popl s% )hs )ms/

LO6 RA L"4OA@ )*)d s+%+*l h*sh%s)o%2*lly . %s *od 1< y*s

@m*ll lympho2y)%2

@m*ll lympho2y)%2, pl*sm*2y)o%d

Moll%2l*, sm*ll 2l*+d 2ll

Moll%2l*, m%5d sm*ll-2l*+d *d l* 2ll

INTRIAT RA L"4OA@ )*)d s+%+*lh*s h%s)o%2*lly . *od E y*s

Moll%2l*, l* 2ll

%((s, sm*ll 2l*+d 2ll

%((s, m%5d sm*ll-2l*+d *d l* 2ll

%((s, l* 2llI RA J%2 d*)h )*)d, .) $ h*+ .2% )hs $%)h 2l*ss%2*l 2hmo)h*py s%2 )h 197


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T RI@ URO4AN-ARIAN !LA@@IMI!ATION OML"4OI NO4LA@@ 0RAL0, l*sd % 1993, $s .*sd o$ $o $%)h d%(()%*)%o m*s/ I)s ps) (oml*)%o %2ldssl%h) *l)*)%os mos) 2)ly % >


39/105

T-2ll polympho2y)%2 lm%*T-2ll l* *l* lympho2y)%2 lm%* )h%s 0!LL0+*%*) 2*ss *ly *m%* C m*o$ .o)F pd*) Am// !l%/ 4*)h/ 13; >89, >


40/105

% lymphopol%(*)%+ d%s*s dsp%) )h 6OF ho$+, poplo )hs md%2%s * po )o d+lop lympho%d hyppl*s%*sod/ 4*)h/ >>; 1E3>, >


41/105

Aod 3


42/105

? 6h%l 6*lds)om's %s )h mos) 2ommo 2*s o( )hhyp+%s2os%)y sydom, yo m*y *lso s %) % som pl*sm* 2llmylom* p*)%)s, o $h )h * )oo m*y d 2lls s*lly% poly2y)hm%* +*, o )oo m*y $h%) 2lls s*lly % 2ho%2myloos lm%*, o o o( )h d%s*ss $%)h $*y )oom*y 2hylom%2os, o $%)h * 2yolo.yl% o +y .*d poly2lo*l*mmop*)hy, o $%)h $*y o )oo m*y pl*)l)s )h *sos *2ompl%2*)d, %+ol+% pl*)l)-do)hl%m %)*2)%os, *dl% )h o)hs, )h .lood po.*.ly $o') . hyp+%s2os % )hl*./

6*lds)om's4%))s.h 4*)holoy !*ss

AL4A A"-!AIN I@A@ o$ mo+d *lo $%)h )ho)h h*+y-2h*% d%s*ss %)o )h pl*ms*-2ll opl*smss2)%o o( )h 6//O/ 2l*ss%(%2*)%o )yp%2*lly *((2)s )h sm*ll.o$l *d %s (*%ly 2ommo % )h N*-*s)/ os) +%2)%ms *yo *dl)s, $ho ps) $%)h m*l*.sop)%o/

{13673}h*+y 2h*% d%s*sF pl*sm*2y)o%d 2lls % %)s)%*l m2os*

? Th%s )*s(oms %)o )h *ss%+ 0d%)***.dom%*l lymphom*0, * B-2ll %mmo.l*s)%2 lymphom*/

{19504}d%)** lymphom*, sm*ll .o$l

Alph* h*+y 2h*%s * *lso pod2d .y ALT lymphom*s/

AA A"-!AIN I@A@ %s * m* (o * mo*ss%+ lymphom* )h*) *lly *((2)s )h ldly/ Loo (o.% )os%ls/

U A" !AIN I@A@ *lly )s lm%2 *ly/

IMMU@ LAR B-!LL L"4OA

Th mos) 2ommo o( )h o-od%'s lymphom*s/
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B(o )%2 po(%l%, $ $ )h*) !O4 2hmo)h*py h*s2d *od D


44/105

? o') $oy *.o) )h d)*%ls (o p*)holo%s)s/ Th * 5)*-*ss%+ 0.l*s)o%d0 *d 0plomoph%20 s.)yps, )2/, )2/,

ALT L"4OA 0m*l)om*0, *md (o %)s o222 o o m2os*ls(*2s, o( 2os %s o$ d(%d .y %)s * )*dm* )*slo2*)%o

)11F18 *d (s%o po)% A4I>CALT1F A/ / 4*)h/ 1>; 1113, >


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MOLLI!ULAR L"4OA@ !BC!! lymphom*

Momly d%+%dd %)o 0sm*ll-2l*+d0, 0m%5d sm*ll-2l*+d *dl* 2ll0 *d 0l*-2ll0 s.2*)o%s, %)'s o$ p))y 2l* )h*)mos) o( )hs lymphom*s * m%5)s o( 2)o.l*s)s l* B-

2lls $%)h o-2l*+d 2l% *d 2)o2y)s 2l*+d B-2lls --.o)h sm.l 2lls % )h m%*l 2)s/ Thy o$ *s odlssodd .y .% lympho2y)s mos)ly T-2lls/

Th 0sm*ll 2lls0 C 2)o2y)s loo l% om*l lympho2y)s52p) (o o o mo 2l()s p )h 2ls 0.))o2 2ll0, )2/,*d )hy l*2 )h m*.ly h)o2hom*)%/

Th 6old *l)h O*%:*)%o h*s * *d% sys)m;

*d 1 ($ )h* E 2)o.l*s)s p h%h po$ (%ld --%dol) d%s*s

*d > -1E 2)o.l*s)s ph h%h po$ (%ld -- %dol)d%s*s

*d 3* mos)ly 2)o.l*s)s $%)h som 2)o2y)s --%dol) d%s*s

*d 3. *lmos) *ll 2)o.l*s)s -- 2ommo, *ss%+d%s*s

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%%)%*lly, .) )d 2 p*)dly *() )*)m) .%s, *2h)%m soo )h* l*s)/

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ph*ps (o )h (%s) )%m *d )h d%s*s .2oms h*d )om**/

{23599}m%5d lymphom*F s )h do)hl%*l 2ll *) >;3< *s * s%: m*{23683}m%5d lymphom*

{23596}odl* l*-2llF *) )h%s po$, Vs) *pp2%*) )h odl*%)y{23581}odl* lymphom*

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Lymphom* odls l*2 )h l%h)-s%d C d*-s%d $ s*llys % m%*l 2)s

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Lymphom* odls ((*2 )h om*l dly%*2h%)2) o( )h lymph od

Lymphom* odls o() o$ .*2-)o-.*2 *d 2o$d o

*o)h, $h%l m%*l 2)s s)*y 2l* o( o *o)h

Lymphom* odls m*y sho$ m*)ls l% om*l m%*l2)s, .) )hy )d )o . )h%

OR L"4OA@
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{23590}d%((s sm*ll 2l*+d lymphom* *ll yo 2* )ll %s )h*) %) %s sm*ll2l*+d{23593}d%((s sm*ll 2l*+d lymphom* *ll yo 2* )ll %s )h*) %) %s d%((s{46344}d%((s sm*ll 2l*+d lymphom*, m*o$

{23581}odl* lymphom*

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ANA4LA@TI! LAR !LL L"4OA A2h/ 4*)h/ L*./d/ 13E; 19, >FE $%)h pod2)%o o( * (s%o pod2) o2oN4CALK, Blood 93; 3


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o5ymooF h%s)%o2y)s * o) lympho2y)s/ Th %mmos)*%%*d 2homosom*l s)d%s $%ll 2l*%(y +y)h%/

{23674}) h%s)%o2y)%2 lymphom*, )s) m

Th old 0%mmo.l*s)%2 lymphom*0 o( )h 6o% !l*ss%(%2*)%o$*s * m%5 o( s+*l d%(() B- *d T-2ll lymphom*s/

MMU@ION L"4OA@, % )h .ody 2*+%)%s $%)ho) * sol%dph*s, * s*lly s % %mmosppssd popl *d *l$*ys* 2*sd .y K@ hps 8 L*2) 3D; 883, 199EFL*2) 3D7; 98< & 1, 199/ K@ %s o$ J%d (o 2*sd(%%)%o !*2 111; >>D, >


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{15446}lymphom*, p%m*y % )h s)om*2h{15542}lymphom*, p%m*y % )h s)om*2h

T-L"4OBLA@TI! L"4OA

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? Ths sm*ll%sh T-2lls h*+ 2o+ol)d %//, mo )h* o2l() 2l%, )hoh )hy * o) *s 2ompl5 *s % @:*ysydom .lo$/ Immolo%s)s o) s%m%l*%)%s $%)h .*.y,%)*-)hym%2 T-2lls/ Th s*l )1DF>1 *d %)s mol2l* .%oloy;4o2/ N*)/ A2*d/ @2%/ 97; 3D97, >

A (*mos B-2ll )mo, )h mos) 2ommo 2h%ldhood 2*2 $hm*l*%* %s dm%2/ I) %s )h (*s)s)-o$% hm* sol%d )mo/os) o(), )h 2h%ldhood +*%*) *%ss % )h V*$/

{46189}A(%2* B%))'s{49035}A(%2* B%))'s

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B%))'s lymphom*4*)%) *d pho)om%2o*ph

KU !oll2)%o

B%))'sBy* L
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B%))'s

N!I6%%md%* !ommos

B%))'s *() )*spl*)


B%))'s@2)%o

6%%md%* !ommos

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NOT; 6'+ *l*dy s )h*) m*y lymphom*s %%mmosppssd p*)%)s, .o)h %s%d *d o)s%d )h

!N@, * s)oly l%d )o )h ps)%-B* +%s / *y,.) o) *ll, h*+ B%))-l% h%s)op*)holoy/ No$*d*ys $2*ll )hs 0pos)-)*spl*)*)%o lymphopol%(*)%+d%sods0, *d )hy )d )o ss %( %mmosppss%o2* . d%s2o)%d/

? Mo som *so, )h K*pos% +%s dos') sm )o2*s pos)-)*spl*) lymphopol%(*)%+ d%s*sAm/ / !l%/ 4*)h/ 131; 3>, >


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.ody m*2oph*s lo*dd $%)h )h%s l%p%d *pp* *s $h%) 0s)*s0**%s) )h .l 0sy0/

Th 0s)*y sy0 *pp**2 o( B%))'s %s * (*+o%) 5*mJs)%o/ s) )o 2o(s yo, )%%.l .ody m*2oph*s*pp* *s s%m%l* 0s)*s0 **%s) )h o)-so-.l-*s-B%))'s0sy0 o( * om*l lymph od/ sp%) 0B% Ro..%s0, )hs)*s o( B%))'s * mo 2osp%2os )h* o)h )%%.l-.ody m*2oph*s .2*s )hy * h*+%ly l*d $%)hl%p%d/

{23620}B%))'s lymphom*, l%p%d dops{46336}B%))'s lymphom*, l%p%d dops{23611}B%))'s lymphom*, ood s)*y sy{46332}B%))'s lymphom*, ood s)*y sy{23641}? B%))'s, m)hyl pyo% )h d 0pyo%oph%l%*0 mly)lls s )h*) )h 2y)opl*sm %s %2h % %.osoms{46326}A(%2* B%))'s, )os%ls

AMRI!AN BURKITT'@, *lmos) *l$*ys B-pos%)%+, %s *lly2*.l $%)h 2hmo)h*py, %( yo 2* ) %) )o )h +%2)%ms/

By 2o)*s), ARI!AN BURKITT'@, * spo*d%2 d%s*s o( yopopl *d )h %mmo2ompom%sd, m*y . B-pos%)%+ oB-*)%+/ I) 2* pod2 m*sss mos) *y$h, *d h*s *$os poos%s/
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"!O@I@ MUNOI@ C @[ZAR" @"NRO L*2) 371; 9DE,>


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o)h$%s-%)*2)*.l T-2ll 2)*os lymphom*/ @ Blood 1


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ps)%-B* hmoph*o2y)%2 sydom

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TRATIN T NON-OKIN'@ L"4OA@; Th%s %s U $s/6'+ *l*dy m)%od som o( )h o+l *)s )h*) * $o%/R%)5%m*. 0R%)5*0, * *)%.ody **%s) !>, >; 8E, >


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? od%'s d%s*s %s * % )h O%)/ Mo som *so,pd%*)%2 od%'s %s 2ommo % )h poo *)%os/

? od%'s d%s*s %s * 2o%:d 2ompl%2*)%o o( AI@, )hohlss )yp%2*l )h* o-od%'s lymphom*s/ No) sp%s%ly,

AI@ p*)%)s $%)h od%'s d%s*s )d )o l*2 lympho2y)s!*2 7; 18E, 1991/

Th m*l%*) 2ll %s )h R-@TRNBR !LL, .) )%l )h l*)s)*s o( )h d%s*s, )h )mo m*sss * 2omposd p%m*%ly o(%(l*mm*)oy 2lls spod% )o )h 2*2/

9ou must reogni@e the&LAI& REED-

TERN7ER% &ELL)

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o multilo1ed

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mirror images o# one

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lymphoyte

predominant su1type4

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{36401}Rd-@). 2ll, o) &F 2y)oloy{40423}Rd-@). 2ll, m%)os%s

od%'s

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NOULAR L"4O!"T 4ROINANT;

I )h%s %llss, )h * sh)s o( lympho2y)s *d somR@-l% 2lls/ o$+, )h R@ 2lls do') + %mmos)*%l% % o)h (oms o( od%'s )hy * !1E-, !DE, B-

2ll m*s * pos%)%+, *d %)'s po.*.ly 0o) *llyod%'s, m*y. * dyspl*s%*0; old $o Blood 87; >D>8,199F Am/ / 4*)h/ 1D; 81>, 199EF d%(() m)*)%osBlood 1


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L"4O!"T 4LTION; os)ly 2*2 2lls, l%))l ls/4o.*.ly )h od%'s 2lls h*+ )* *dd%)%o*l m)*)%os/

? Th .*2od m*y . lo)s o( pooly-$o+ 2oll*0d%((s (%.os%s +*%*)0 o Vs) )%2l% 0)%2l* +*%*)0,

$%)h $%ldly **pl*s)%2 2lls/

Th d%s*s o() .) o) *l$*ys ps)s *) l*) s)*/

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{23524}lympho2y) dpl)d od%'s d%s*s/ s) pl*% **pl*s)%2/

NOULAR @!LRO@I@; Th%s (*)s l*2* Rd-@).+*%*)s *d * )d2y (o )h ls%o )o .2om 2%ss2ossd .yds 2oll* .*ds/ Th poos%s %s *lly ood/

{23542}odl* s2los% od%'s d%s*s{23545}odl* s2los% od%'s d%s*s{23548}l*2* Rd-@). +*%*)s{23551}l*2* Rd-@). +*%*)

Nodl* s2los% od%'sBy* L Nodl* s2los% od%'s

6.4*)h 4ho)o

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L*2* 2lls

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od%'s d%s*s %s d)m%d .y s)*, *)h )h* .y )yp/Almos) *ll p*)%)s $%)h s)* I o IIA d%s*s * o$ 2d/ Th%sdops )o *od E>, >/

R-@TRNBR ARIANT@ * *lso m*l%*)/

ONONU!LAR R-@TRNBR-LIK !LL@ 0od%2lls0 h*+ s%l-lo.d 2l% *d o 2lols/ Thy m*y .s % *y +*%*) o( od%'s d%s*s/

? L4 !LL@ ? 0L& 2lls0 h*+ s2*)y 2y)opl*sm, .% o..y2l%, *d sm*ll 2lol%/ Thy * s % lympho2y)pdom%*2 od%'s d%s*s/

LA!UNAR R-@TRNBR !LL@ h*+ *.d*), p*l

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60/105

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0B0 m*s (+, $%h) loss G1


61/105

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62/105

3AMI4

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63/105

{23842}*2) lympho2y)%2 lm%*, .*%{08734}*2) lm%*, l%+F *s yo $old 5p2), )h lm%* %s .l{08735}*2) lm%*, l%+{08736}*2) lm%*, l%+

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64/105

RUL; Ay lm%* o lymphom* 2* %+ol+ )h l%+,.) l*m) %s s*lly o) sp2)*2l*/ od%'s *do-od%'s lymphom*s $%ll (%s) *pp* % )h po)*l**s/

L"4OI LUKIA@ 2*ll )h +*%os %ds o( om*llympho2y)s, *d "LOI 0myloos0, o .)), 0*lo2y)%20lm%*s 2*ll * om*l *lo2y)/

"o mm. )h*) "LO!"T@, )h p2sos o(*lo2y)s, * )h mos) 2ommo 2ll % om*l .o m*o$"LO-F h2 )h% *m/

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p*)%)s *) %s (o o)/ Th %s %2*ss ()h $h 2*2 2lls *dy% .y )h pod d% )h*py/ Th )hoh)(l o2olo%s) %+spophyl*2)%2 md%2*)%o/

A!UT L"4OBLA@TI! LUKIA 0ALL0F d%*os%s Am/ / !l%/4*)h/ 111; D7, 1999

A2) lympho.l*s)%2 lm%*Bo m*o$

6.4*)h 4ho)o

A2) lympho.l*s)%2 lm%*4%ph*l sm*

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65/105

ALL sms )o s)% *) *dom/ R*d%*)%o 5pos %s * o$ %s(*2)o, o$'s sydom %ds * *) 1E5 )h om*l %s, * p*)%)'s%d)%2*l )$% h*s * >97, 199FL*2) 3D9; 3DD, 1997/

R*2hl !*so's (o)*) %//, (*ls *d %(l*mm*)oyd%s2ss%o o( lm%* % 0@%l) @p%0 %s o$ h%s)oy/ @h $*s%h) *.o) )h d* o( T )o .%ds' s, *d h m*%pl*)%oo( )h d*)* o lm%* %s * m%o .lo) o )h mmoy o( * *)s2%2 $%)/ No.ody's p(2)/ Tod*y, )h's o s%osd%s2ss%o o( )h old *lld l% .)$ %s2)%2%d 5pos*d lm%* ? )h o p*p I 2old (%d (om )h d2*d 1997->; 1D=/// B% 2lls, lo)s o( 2l()s, o() 2lol%F mo 2ommo %*dl)s

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66/105

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67/105

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68/105

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69/105

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70/105

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? LL *lso (oms * (s%o pod2) $%)h N-1 $h%2h %s *dV*2)*d * s2-l% o 2homosom 19 4o2/ N*)/ A2*d/ @2%/ 9D;>E3, 1999 % )11F19 lm%*sF )h%s (s%o pod2) %h%.%)s pE3'spod2) Blood 93; 3>1, 1999/
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71/105

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72/105

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73/105

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75/105

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76/105

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77/105

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78/105

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79/105

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80/105

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82/105

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83/105

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84/105

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85/105

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86/105

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87/105

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88/105

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89/105

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90/105

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92/105

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93/105

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94/105

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95/105

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96/105

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97/105

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98/105

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99/105

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100/105

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@plom*lyU.** A)l*s o( 4*)holoy

@4TI! @4LN 0osp2%(%2 *2) spl%)%s0 %s )yp%2*l o( s%os .*2)%*l%(2)%os/ Lo*dd $%)h polys *d *.om*lly so(), )h old om) p*)holo%s)sm*d )h 2omp*%so )o 0)om*)o p*s)0, $h%2h %s +y m2h sm.ls/

5*2)ly $hy )h spl .2oms l% )h%s % d*)hs (om sps%s, *d +*y)h% ls, m*%s *s mys)%os *s sps%s %)sl(/ "o'll s po(odloss o( )h B-2lls *d T-hlp 2lls *od )h $h%) plp, *d *pop)os%so( )h dd%)%2 )%2l* 2lls )h*) m*%)*% )h s)2) o( )h splN 3D8; 138, >


101/105

INMAR!T@ * 2ommo % )h spl, *d m*y sl) (om *)hom.ol%:*)%o)h )$%s)y spl%2 *)y %s )h mos) s+ly *((2)d % )h .ody, l()-s%dddo2*d%)%s, o %(%l)*)%+ d%s*s/

I(*2)s6.4*)h 4ho)o

? N2os%s % * .lood-.lo*)d spl )yp%2*lly, % s%2ls %s l%ly )opod2 %o- *d 2*l2%m-%2h s2*s 2*lld ANA-AN" BOI@/Th 0*)ospl2)om%:d0 spl o( * old s%2l-2ll d%s*s p*)%) %smos)ly 2omposd o( s2h s2*s/

@%2l 2ll d%s*s

A)ospl2)omy6.4*)h 4ho)o

4RIAR" NO4LA@@ o( )h spl * 2ommo/ B% )mos **lmos) + o( *y %mpo)*2/ Ay lymphom* o do)hl%*l opl*sm 2**%s h/ TA@TA@@ )o )h spl * 5p2)d % mos) lm%*s *dod%'s *d o-od%'s lymphom*s, .) 2*2%om*s *d s*2om*s +ysldom o$ % )h spl/

RU4TUR @4LN sl)s (om .lo$s h*d %( yo' h*l)hy, l%h) .lo$s

s((%2 (o )hos $%)h %(2)%os moo2los%sF mm. !4R *s * 2*s B/d/ / 3>>; D8


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"o mm. )h d%((2 .)$ s2)%os *d sm*s, %h)

? @LI! OM LIM RI6; BLOO !LL@17E` *m%*, %o d(%2%2yF om*l_1371E` lympho2y), om*l_1388` d .lood 2ll, om*l .lood_1391


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_1D7>>` )%2lo2y)s, om*l_1D7>3` )%2lo2y)s, om*l_1D7>D` d .lood 2ll, *.om*l_1D7>E` d .lood 2ll, *.om*l_1D7>` pl*)l)s, om*l

_1D7>7` pl*)l)s, om*l_1D7>8` poomo.l*s), om*l_1D7>9` poomo.l*s), om*l_1D73` omo.l*s)_1D733` omo.l*s)_1D73D` polymopho2l* lo2y) & ? lympho2y)_1D73E` polymopho2l* lo2y) & ? lympho2y)_1D73` omo.l*s) s%s_1D737` omo.l*s) s%s l*.lld

_1D738` mylo2y), om*l_1D739` mylo2y), om*l_1D7D` mylo2y), .*d (om_1D7D3` mylo2y), .*d (om_1D7DD` mylo2y), om*l_1D7DE` mylo2y), om*l_1D7D` mylo2y), om*l_1D7D7` mylo2y), om*l_1D7D8` mylo2y), om*l_1D7D9` mylo2y), om*l_1D7E` mylo2y) & m**yo2y), om*l_1E193` pl*sm* 2ll, #>3_1E>` polymopho2l* lo2y), om*l_>>3


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? @LI! OM LIM RI6; L"4OI ORAN@_117ED
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105/105

_1E19E` spl, ? d plp *d $h%) plp_1E19` spl, 2)*l *)y_1E197` spl, 2)*l *)y *d m%*l 2)_1E198` spl, )*.2l*_1E199` )hyms, #>E

_1E>7` )os%l p*l*)%
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